AB0542 PRIMARY SJÖGREN’S SYNDROME AND INTERSTITIAL LUNG DISEASE. RESULTS OF AN OBSERVATIONAL COHORT
Authors: P. Muñoz Reinoso, F. J. Toyos Sáenz de Miera, I. M. Fernández-Maeztu Palacín, A. Fernández Reboul Fernández, J. J. Pérez Venegas
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Primary Sjögren's Syndrome (pSS) is a chronic systemic autoimmune disease of unknown etiology, with an estimated prevalence of 0.25% (0.15-0.43). It has a broad clinical spectrum and extraglandular manifestations such as Interstitial Lung Disease (ILD), being one of the main contributors to morbidity and mortality in patients with pSS <sup>[1]</sup>. <h3>Objectives</h3> Analyze the clinical characteristics of patients with pSS and ILD, describe the patterns of lung involvement and the evolution of the disease as well as the treatments that were used. <h3>Methods</h3> Observational, retrospective, single-center study, in the <i>Hospital Universitario Virgen Macarena</i> Rheumatology department, from 2010 to 2022, including patients with pSS according to EULAR/ACR criteria and ILD. The data obtained from the medical records was analyzed by the statistical software SPSS v28. This study has been approved by the CEIC. <h3>Results</h3> 19 patients were included, 17 (89.5%) women, with a mean age of 66.4 years (62-71). All presented antinuclear and anti-SSA antibodies, 31.6% (6) anti-SSB antibodies and 43.8% (7) Rheumatoid Factor, 63.2% (12) hypergammaglobulinemia and none hypocomplementemia. 36.8% (7) were former smokers. The extraglandular manifestations and comorbidities are shown in Table 1. The mean age at diagnosis of ILD was 60.1 years (53.3-65.8). The time elapsed until the diagnosis was 93 months (12-198), however, in 52.6% (10) the diagnosis of ILD was prior to pSS diagnosis for approximately 17 months (6-27). The most common radiological pattern with 63.2% (12) is non-specific interstitial pneumonia, two patients presented lymphocytic interstitial pneumonia, two usual interstitial pneumonia, one organizing pneumonia and the rest of them other patterns. Two patients required lung biopsy. Prior to diagnosis, 3 patients were being treated with hydroxychloroquine, the rest had not required treatment with immunomodulators. Once diagnosed, 52.6% (10) required immunosuppressants (Graph 1). 80% (8) continue in treatment, 7 with mycophenolate mofetil with a mean duration of 48 months (20-75). 47.4% (9) required oral glucocorticoids at some point. 88.2% (15) improved or remained radiologically stable by HRCT, 73.3% (11) according to respiratory function tests (decrease in FVC &lt;10% and DLCO &lt;15%) and 72% (13) dyspnea remained stable or improved according to the MRC scale, with no significant differences between patients with and without treatment. The mean evolution time of ILD was 77.3 months (63-91). There were 2 deaths, one due to progression of the disease and the other due to a head trauma. <h3>Conclusion</h3> Interstitial lung disease is an extraglandular manifestation present in patients with pSS, therefore being essential the early diagnosis. In our case, after the diagnosis of ILD, half of the patients required immunosuppressive treatment to prevent the progression of the disease, with a large number of patients remaining currently stable. <h3>Reference</h3> [1]Gao H, Sun Y, Zhang XY, Xie L, Zhang XW, Zhong YC, et al. Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease. Medicine (Baltimore). 2021 Sep 3;100(35):e26777. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.